Carcinoid Research Articles

Multiomic sequencing of paired primary and metastatic small bowel carcinoids

Background: Small bowel carcinoids are insidious tumors that are often metastatic when diagnosed. Limited mutation landscape studies of carcinoids indicate that these tumors have a relatively low mutational burden. The development of targeted therapies will depend upon the identification of mutations that drive the pathogenesis and metastasis of carcinoid tumors. Methods: Whole exome and RNA sequencing of 5 matched sets of normal tissue, primary small intestine carcinoid tumors, and liver metastases were investigated. Germline and somatic variants included: single nucleotide variants (SNVs), insertions/deletions (indels), structural variants, and copy number alterations (CNAs). The functional impact of mutations was predicted using Ensembl Variant Effect Predictor. Results: Large-scale CNAs were observed including the loss of chromosome 18 in all 5 metastases and 3/5 primary tumors. Certain somatic SNVs were metastasis-specific; including mutations in ATRX, CDKN1B, MXRA5 (leading to the activation of a cryptic splice site and loss of mRNA), SMARCA2, and the loss of UBE4B. Additional mutations in ATRX, and splice site loss of PYGL, leading to intron retention observed in primary and metastatic tumors. Conclusions: We observed novel mutations in primary/metastatic carcinoid tumor pairs, and some have been observed in other types of neuroendocrine tumors. We confirmed a previously observed loss of chromosome 18 and CDKN1B. Transcriptome sequencing added relevant information that would not have been appreciated with DNA sequencing alone. The detection of several splicing mutations on the DNA level and their consequences at the RNA level suggests that RNA splicing aberrations may be an important mechanism underlying carcinoid tumors.

Periampullary Adenocarcinoma with Incidental Gastric Carcinoid Tumor: Report of an Extremely Rare Case

Periampullary neoplasms are a heterogeneous group of tumors arising within 2 cm of the ampulla of Vater. Neuroendocrine tumors can originate throughout the entire body from neuroendocrine cells. These neoplasms exhibit deep differences, according to their origin and biological behavior. The synchronous presence of a primary tumor in patients affected by a neuroendocrine tumor is reported in the literature; incidence is variable and the most common site is the gastrointestinal tract. Here we report a case of 68 years old female presented with jaundice, anorexia and weight loss. She was mildly anaemic. Gall bladder was palpable. There was no organomegaly. Her CT scan and MRCP revealed a growth in periampullary region. So she underwent Whipple’s procedure. Histopathological report showed synchronous presence of periampullary carcinoma and a carcinoid tumor in stomach. As the carcinoid tumor was nonfunctioning and very small in size, it was not identified in pre-operative work up. J Shaheed Suhrawardy Med Coll 2021; 13(2): 183-186

Prognosis of Unresected vs Resected Small Pulmonary Carcinoid Tumors

BackgroundPrevious studies have shown that overall survival after lung resection for pulmonary carcinoid tumors is favorable. It is unclear what the prognosis is for observation rather than resection for small carcinoid tumors. MethodsWe queried the National Cancer Database to identify patients presenting with primary pulmonary carcinoid tumors between 2004 and 2017. We included patients with small (<3 cm) primary pulmonary carcinoids, who were observed or underwent a lung resection. To minimize confounding by indication, we used propensity score matching, while accounting for age, sex, race, insurance type, Charlson-Deyo comorbidity score, typical and atypical histology, tumor size, and year of diagnosis. We used Kaplan-Meier survival analyses to compare 5-year overall survival in the matched cohorts. ResultsOf 8435 patients with small pulmonary carcinoids, 783 (9.3%) underwent observation and 7652 (91%) underwent surgical resection. After propensity score matching, surgical resection was associated with improved 5-year overall survival (66% vs 81%, P < .001). No significant difference in overall survival was found between wedge and anatomic resection (88% vs 88%, P = .83). In patients undergoing resection, lymph node sampling at the time of wedge and anatomic resection increased 5-year overall survival (90% vs 86%, P = .0042; 88% vs 82%, P = .04, respectively). ConclusionsSurgical resection of small pulmonary carcinoids is associated with improved survival compared with observation. When surgical resection is performed, wedge and anatomic resection result in similar survival, and lymph node sampling improves survival.

Improving Small Lesion Segmentation in CT Scans using Intensity Distribution Supervision: Application to Small Bowel Carcinoid Tumor.

Finding small lesions is very challenging due to lack of noticeable features, severe class imbalance, as well as the size itself. One approach to improve small lesion segmentation is to reduce the region of interest and inspect it at a higher sensitivity rather than performing it for the entire region. It is usually implemented as sequential or joint segmentation of organ and lesion, which requires additional supervision on organ segmentation. Instead, we propose to utilize an intensity distribution of a target lesion at no additional labeling cost to effectively separate regions where the lesions are possibly located from the background. It is incorporated into network training as an auxiliary task. We applied the proposed method to segmentation of small bowel carcinoid tumors in CT scans. We observed improvements for all metrics (33.5% → 38.2%, 41.3% → 47.8%, 30.0% → 35.9% for the global, per case, and per tumor Dice scores, respectively.) compared to the baseline method, which proves the validity of our idea. Our method can be one option for explicitly incorporating intensity distribution information of a target in network training.

Fully-automated detection of small bowel carcinoid tumors in CT scans using deep learning.

Small bowel carcinoid tumor is a rare neoplasm and increasing in incidence. Patients with small bowel carcinoid tumors often experience long delays in diagnosis due to the vague symptoms, slow growth of tumors, and lack of clinician awareness. Computed tomography (CT) is the most common imaging study for diagnosis of small bowel carcinoid tumor. It is often used with positron emission tomography (PET) to capture anatomical and functional aspects of carcinoid tumors and thus to increase thesensitivity. We compared three different kinds of methods for the automatic detection of small bowel carcinoid tumors on CT scans, which is the first to the best of ourknowledge. Thirty-three preoperative CT scans of 33 unique patients with surgically-proven carcinoid tumors within the small bowel were collected. Ground-truth segmentation of tumors was drawn on CT scans by referring to available 18 F-DOPA PET scans and the corresponding radiology report. These scans were split into the trainval set (n = 24) and the test positive set (n= 9). Additionally, 22 CT scans of 22 unique patients who had no evidence of the tumor were collected to comprise the test negative set. We compared three different kinds of detection methods, which are detection network, patch-based classification, and segmentation-based methods. We also investigated the usefulness of small bowel segmentation for reduction of false positives (FPs) for each method. Free-response receiver operating characteristic (FROC) curves and receiver operating characteristic (ROC) curves were used for lesion- and patient-level evaluations, respectively. Statistical analyses comparing the FROC and ROC curves were alsoperformed. The detection network method performed the best among the compared methods. For lesion-level detection, the detection network method, without the small bowel segmentation-based filtering, achieved sensitivity values of (60.8%, 81.1%, 82.4%, 86.5%) at per-scan FP rates of (1, 2, 4 ,8), respectively. The use of the small bowel segmentation did not improve the performance ( ). For patient-level detection, again the detection network method, but with the small bowel segmentation-based filtering, achieved the highest AUC of 0.86 with a sensitivity of 78% and specificity of 82% at the Youdenpoint. The carcinoid tumors in this patient population were very small and potentially difficult to diagnose. The presented method showed reasonable sensitivity at small numbers of FPs for lesion-level detection. It also achieved a promising AUC for patient-level detection. The method may have clinical application in patients with this rare and difficult to detectdisease.

Primary lung cancer in children and adolescents: Analysis of a surveillance, epidemiology, and end results database.

The incidence of primary lung cancer (LC) in children and adolescence was rare. We analyzed data from a SEER database to better define the incidence, clinical characters, pathology, treatment, and outcomes of rare primary malignant pulmonary tumors in childhood and adolescence. Patients were chosen from the SEER database (SEER*Stat 8.4.0 software) from 2000 to 2019 and all patients were pathologically diagnosed with primary malignant tumors of the lung and bronchus. Demographic characteristics of patients (age, gender, race, primary site, laterality, location, differentiation grade, operation methods, histology, and history of radiotherapy and chemotherapy), as well as TNM stage and survival time, were collected. A total of 301 cases of children ≤19 years of age with a primary malignant pulmonary tumor were reported to the SEER database from 2000 to 2019. There were 143 men (47.5%) and 158 women (52.5%). Whites represented majority of patients (79.7%), followed by Black (13.6%) and others (6.7%). As for the primary site, the main site was the lower lobe (33.2%), followed by the upper lobe (26.9%). Most of the patients (80.4%) underwent surgery. Lobectomy (39.9%) is the main operation method. Only 28 (9.3%) patients received radiotherapy and 112 (37.2) patients received chemotherapy. Carcinoid tumor was the most common histology (29.6%), followed by pulmonary blastoma (PB) (22.3%), mucoepidermoid carcinoma (MEC) (12.3%), adenocarcinoma (10.3%), neuroendocrine tumor (NET) (5.7%), squamous cell carcinoma (SCC) (5.3%), atypical carcinoma (2.3%). The mean follow-up time was 100 months. For the entire group of children and adolescents, the 1-year OS was 89.1%, and the 3-year overall survival (OS) was 79.7%. the 5-year OS was 77.9%, the 10-year OS was 75.7%, and the 15-year OS was 73.9%. And 1-year lung cancer specificity survival (LCSS) was 89.8%, and the 3-year LCSS was 80.4%. the 5-year LCSS was 79.4%, the 10-year LCSS was 77.7%, and the 15-year LCSS was 75.9%. The OS of atypical carcinoma, carcinoid tumor, and MEC were in the top three. Primary LC in children and adolescent were rare and histopathological diverse. Fortunately, children and adolescents with LC had an overall favorable outcome after treatment. Histology, differentiation grade, surgery, TNM stage, and therapeutic modalities have important influence on OS. The further treatment experience of each pathological type would make better evidence-based practice possible.

Typical Carcinoid Tumor Cases Causing Ectopic ACTH Syndrome: Dramatic Response to Surgery.

Although Cushing's Syndrome (CS) is usually caused by pituitary/adrenal adenomas, in the remaining few cases, Adrenocorticotrophic hormone (ACTH) is secreted by extrapituitary neuroendocrine tumors (NET). In typical pulmonary / bronchial carcinoid tumors leading to ectopic ACTH syndrome (EAS). The main principle of treatment is the localization of the ACTH-secreting tumor and its surgical removal. In this case report, we aimed to present two typical carcinoid cases, whose ACTH and cortisol levels returned to normal after lung surgery for ectopic ACTH. One of the cases, a 32-year-old female patient with CS symptoms and signs, was referred to our department after detecting a lesion in the left lower lobe in the thorax CT, which did not show an obvious focus on cranial MRI. The other patient, a 36-year-old male, had previously undergone adrenalectomy for Cushing's syndrome and was admitted to the emergency department due to adrenal insufficiency. The lesion seen in the right lower lobe on thorax CT was evaluated as an ectopic ACTH focus. After the tumors of the patients were resected according to surgical principles, CS clinic regressed, and ACTH and cortisol values returned to normal. Pathological examinations of the surgically resected tumors were reported as typical carcinoid. With surgically removed typical carcinoids tumors, excellent longterm survival results can be achieved and hypercortisolism can be controlled.

223P Surgery versus bronchoscopic cryotherapy for the treatment of lung carcinoid tumors: An overview of 5-year experience

223P Surgery versus bronchoscopic cryotherapy for the treatment of lung carcinoid tumors: An overview of 5-year experience

Mediastinal teratoma with double malignant transformation

We report a case of a 65-year-old woman with retrosternal discomfort, dyspnea, and weight loss in whom the computed tomography showed a multicystic mass with fat content and calcified wall in the anterior mediastinum. After surgical resection, pathological examination revealed synchronous primary colorectal adenocarcinoma and primary carcinoid tumor arising in a mediastinal mature cystic teratoma. Treatment with oxaliplatin and capecitabine was instituted, and subsequent checkups showed no evidence of recurrence. Malignant transformation of mature cystic teratoma is a rare complication that occurs in 1–3% of these tumors and consists of a nongerm cell malignant tumor arising from a preexisting mature teratoma. Any of the constituent tissues of a teratoma has the potential to undergo malignant transformation. In case of limited disease, treatment should be surgical. There is no consensus regarding adjuvant treatment, and the existence of a double neoplastic lineage may complicate the choice of chemotherapy treatment.

Clinical profile, surgical management and outcome of bronchial carcinoids - a single centre experience

Background and objectives: Bronchial carcinoid tumors are neuroendocrine neoplasms that range from low-grade typical carcinoids to more aggressive atypical carcinoids and, therefore demonstrate a wide spectrum of clinical behaviors, histologic features and outcome. The aim of the present study was to investigate the clinical profile, surgical management and outcome of bronchial carcinoids at a single centre over two years period. Materials and methods: Patients with a final histologic diagnosis of bronchial carcinoid tumor were included in the study. Evaluation comprised of clinical history and physical examination, postero-anterior and lateral chest radiographs, and computed tomographic (CT) scans of the chest and upper abdomen (including liver and adrenal glands). Performance status was assessed by the Karnofsky scale. Pulmonary function tests were performed routinely. Results: A total of 18 patients were included in the study. Out of 18 cases, 10 (55.6%) were female and 8 (44.4%) were males. Sixteen (88.9%) patients had typical carcinoid tumor and 2 (11.1%) had atypical carcinoid tumor. The tumor was located in the right lung in 11 (61.1%) and in the left lung in 7 patients (38.9%). Surgeries included 15 standard lobectomies and 3 bronchial sleeve resection. At one month post surgery, there was 13-22% increase in post operative FEV1 in patients who underwent bronchial sleeve resection while in patients who underwent lobectomy, the post operative FEV1 was 84% of pre-operative FEV1. Post surgery, all patients were in group A as per Karnofsky performance status. Conclusion: Standard care of bronchial carcinoid tumors is surgical resection, and the surgical approach should depend on tumor’s size, location and histology. IMC J Med Sci. 2023; 17(2):003. DOI: https://doi.org/10.55010/imcjms.17.013 *Correspondence: Farooq Ahmad Ganie, Department of Cardiovascular and Thoracic Surgery, SKIMS, Soura, Srinagar, Kashmir, India. Email: farooq.ganie@ymail.com

Clinical characteristics and endoscopic findings in autoimmune gastritis – A retrospective study

Abstract Objectives: Autoimmune gastritis (AG) is a rare condition that increases the risk of developing stomach adenocarcinomas or carcinoid tumours. The objectives of the present research were to summarise the clinical traits of AG patients, together with gastroscopic and histopatho-logic findings, demographic data, and hematologic characteristics. Patients and methods: A medical centre assessed 58 AG patients from January 2019 to December 2022. Results: The majority of the patients were female (73.7%), and the mean age of the participants at the time of the diagnosis was 57.7 ± 12.1 years. We identified pernicious anaemia (54.4%), iron deficiency anaemia (21.1%), as well as autoimmune disorders (96.5%). Though 78.9% of patients reported having gastrointestinal symptoms, 69% presented exclusively upper gastrointestinal symptoms, 17% only had lower, and 14% had concurrent upper and lower gastrointestinal symptoms. All 58 AG patients were examined for associated gastric lesions, although abnormal injuries were detected in only 22 of them. One patient (1.8%) had adenocarcinoma, while five patients (8.8%) had type 1 neuroendocrine tumours (NET). In addition, hyperplastic polyps were found in 16 (28.1%) individuals. Conclusions: Other autoimmune diseases were present with AG, which showed a female predominance. Clinicians should give AG more significant thought by allowing access to interdisciplinary teams.

Incidental diagnosis of appendiceal neuroendocrine (carcinoid) tumors during benign gynecologic surgery: a case series

Incidental diagnosis of appendiceal neuroendocrine (carcinoid) tumors during benign gynecologic surgery: a case series

Synthesis and Pharmacological Evaluation of the derivatives of 1,4-dihydropyridine: A Pleiotropic Molecule with Antiulcer activity

As previously researches indicate that 1,4-Dihydropyridine is a versatile molecule and it is clearly shown that it possesses different therapeutic activity in versatile disease. It shows different activity such as anticancer, anticonvulsant, anticoagulant, anti-alzheimer, antitubercular and antiulcer. Various adverse effects of proton pump inhibitors and H2 blockers were reported by many Investigators. These are Gastric cancer, Colon cancer, Gastric carcinoid tumor, gastrointestinal infection and many others. A good antiulcer potential of 1,4 Dihydropyridine with aromatic primary amine substitution seen here, with that reason our research work to conduct synthesis of 1,4 dihydropyridine derivative by replacement with some other aromatic primary amine scaffolds, and to evaluate the antiulcer potency of 1,4 Dihydropyridine. All chemicals were purchased from verified companies. Characterization of these synthesized molecules were performed by various spectroscopic methods and evaluated for in-vivo antiulcerogenic potential by using different animal models. A series of approximately in 25 numbers 1, 4 Dihydropyridine derivatives were synthesized and characterized on their spectroscopic studies. Antiulcer activity was performed in ethanol induced gastric lesions followed with five different models. In series of approximately 25 derivatives synthesized compound A5, A6, B5 and B6 shows significant activity in comparison to standard drug i.e.Omeprazole and among all compound, A5 and B6 (30mg/kg) shown potent antiulcer activity. Our final remark clearly stated that 1,4 Dihydropyridine previously established as calcium channel blocker, must shown antiulcer potential and all synthesized compound can serve as leads for new antiulcer agents after further investigation.

Emerging Immunotherapeutic and Diagnostic Modalities in Carcinoid Tumors.

Evasion of innate immunity represents a frequently employed method by which tumor cells survive and thrive. Previously, the development of immunotherapeutic agents capable of overcoming this evasion has realized pronounced clinical utility across a variety of cancer types. More recently, immunological strategies have been investigated as potentially viable therapeutic and diagnostic modalities in the management of carcinoid tumors. Classic treatment options for carcinoid tumors rely upon surgical resection or non-immune pharmacology. Though surgical intervention can be curative, tumor characteristics, such as size, location, and spread, heavily limit success. Non-immune pharmacologic treatments can be similarly limited, and many demonstrate problematic side effects. Immunotherapy may be able to overcome these limitations and further improve clinical outcomes. Similarly, emerging immunologic carcinoid biomarkers may improve diagnostic capabilities. Recent developments in immunotherapeutic and diagnostic modalities of carcinoid management are summarized here.

Artificial intelligence-augmented analysis of contemporary procedural, mortality, and cost trends in carcinoid heart disease in a large national cohort with a focus on the "forgotten pulmonic valve".

Carcinoid heart disease is increasingly recognized and challenging to manage due to limited outcomes data. This is the largest known cohort study of valvular pathology, treatment (including pulmonary and tricuspid valve replacements [PVR and TVR]), dispairties, mortality, and cost in patients with malignant carcinoid tumor (MCT). Machine learning-augmented propensity score-adjusted multivariable regression was conducted for clincal outcomes in the 2016-2018 U.S. National Inpatient Sample (NIS). Regression models were weighted by the complex survey design and adjusted for known confounders and the likelihood of undergoing valvular procedures. Among 101,521,656 hospitalizations, 55,910 (0.06%) had MCT. Patients with MCT vs. those without had significantly higher inpatient mortality (2.93 vs. 2.04%, p = 0.002), longer mean length of stay (12.20 vs. 4.62, p < 0.001), and increased mean total cost of stay ($70,252.18 vs. 51,092.01, p < 0.001). There was a step-wise increased rate of TVR and PVR with each subsequent year, with significantly more TV (0.16% vs. 0.01, p < 0.001) and PV (0.03 vs. 0.00, p = 0.040) diagnosed with vs. without MCT for 2016, with comparable trends in 2017 and 2018. There were no significant procedural disparities among patients with MCT for sex, race, income, urban density, or geographic region, except in 2017, when the highest prevalence of PV procedures were performed in the Western North at 50.00% (p = 0.034). In machine learning and propensity score augmented multivariable regression, MCT did not significantly increase the likelihood of TVR or PVR. In sub-group analysis restricted to MCT, neither TVR nor PVR significantly increased mortality, though it did increase cost (respectively, $141,082.30, p = 0.015; $355,356.40, p = 0.012). This analysis reflects a favorable trend in recognizing the need for TVR and PVR in patients with MCT, with associated increased cost but not mortality. Our study also suggests that pulmonic valve pathology is increasingly recognized in MCT as reflected by the upward trend in PVRs. Further research and updated societal guidelines may need to focus on the "forgotten pulmonic valve" to improve outcomes and disparities in this understudied patient population.

Clinical Diagnosis and Treatment of Primary Laryngeal Neuroendocrine Carcinoma.

This study aims to investigate the clinical diagnosis and treatment characteristics of primary laryngeal neuroendocrine carcinoma. A retrospective analysis was performed for the data of 6 patients with primary laryngeal neuroendocrine carcinoma admitted to the Department of Otorhinolaryngology of Dalian Central Hospital from September 2015 to January 2022, all of whom were male patients. And laryngoscopy showed that 4 had a similar appearance to hemangioma, 1 had nodular hyperplasia, and 1 was papilloma-like. The site of onset was located on the laryngeal surface of the epiglottis in 3 cases, situated in the aryepiglottic fold in 2 cases, and the posterior ventricular zone in 1 case. Five patients underwent surgery, including 4 by transoral endoscopic cryogenic plasma radiofrequency mass resection; one underwent a cervical lymph node dissection and supraglottic horizontal hemilaryngectomy. The remaining 1 case was treated in another hospital after the pathological diagnosis was confirmed by biopsy. All patients had no cervical lymph node metastases and were not supplemented with chemoradiotherapy. The postoperative pathological diagnosis of all 5 patients was neuroendocrine carcinoma. No cancer cells were seen at the margins and base. One patient was lost to follow-up after treatment in another hospitals and the rest were followed up for 6months to 6years. One patient had removed laryngeal tumor and epiglottis by a low-temperature plasma radiofrequency ablation, recurrence 1year after surgery, and no recurrence for 5years after supraglottic horizontal hemilaryngectomy combined with prophylactic cervical lymph node dissection, no recurrence for the other 4patients after 6months to 4years of follow-up. The common sites of laryngeal neuroendocrine carcinoma are the laryngeal surface of the epiglottis and the aryepiglottic fold. The appearance of the tumor is mainly similar to that of hemangioma. Moreover, a common pathological classification is atypical carcinoid tumors, where early detection and surgical treatment can achieve a good therapeutic effect.

Frequency of Pulmonary Carcinoid Tumor Research Presented at Major International Neuroendocrine and Lung Cancer Scientific Meetings

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Hedinger Syndrome Secondary to Carcinoid Tumor of the Ovary: Anesthetic Challenge and Echocardiographic Illustration

Hedinger Syndrome Secondary to Carcinoid Tumor of the Ovary: Anesthetic Challenge and Echocardiographic Illustration

A 5 Year Tertiary Centre Experience of Frequency &amp; Risk Factors Association with Primary and Secondary Lung Cancers

Introduction: Lung cancer is one of the commonest cancers worldwide having either small cell or non-small cell types and smoking is its major risk factor. We intend to study distribution of various lung malignancies and their association with various risk factors in a Pakistani population sample.&#x0D; Aims &amp; Objectives: To evaluate the frequency of types of primary and secondary lung cancers in a sample of the Pakistani population and see their association with age, gender, and smoking.&#x0D; Place and duration of study: The department of Pulmonology, Shaikh Zayed Hospital, Federal Postgraduate Medical Institute, Lahore, Pakistan, from August 2017 to August 2022 (5 years).&#x0D; Material &amp; Methods: Analysis of retrospective data of 156 consecutive patients age 40-70 years diagnosed with primary or secondary lung cancers. Data were stored and analyzed using IBM-SPSS version 23.0. Diagnosis was established through fibreoptic (FOB), or rigid bronchoscopies or CT and U/S guided percutaneous lung biopsies, P- value&lt;0.05 was taken as significant&#x0D; Results: There were 156 samples, among them 37.8% were found with age group 41 – 60 years old, 31.4% with age group 61 – 70 years old, mean age of subjects was 58.3 (SD=±14.9) years. 82.1% were of male gender, 42.9% were non- smokers, 25% were found with less than or equal to 25 smoking pack years, median smoking pack years were 30 with range 5 – 100 smoking pack years. Distribution of primary lung malignancy revealed; 28.8% having Squamous cell carcinoma, 35.3% with Adenocarcinoma, 13.5% with small cell carcinoma, 2.6% with large cell carcinoma and 5.8% with primary lung carcinoid tumors. Secondary deposits of cancer in lungs were found in 22 (14.1%) subjects. Pearson Chi Square test showed significant association of primary type of cancer with age group, gender, and smoking pack years (p &lt;0.01).&#x0D; Conclusion: Adenocarcinoma was most frequent lung malignancy followed by Squamous cell carcinoma and small cell carcinoma was less frequent. Advancing age, male gender and cigarette smoking are strongly associated with the diagnosis of lung cancer.

Contemporary outcomes of esophageal and gastroesophageal junction neuroendocrine tumors.

Adenocarcinoma and squamous cell esophageal cancers have been extensively studied in the literature. Esophageal neuroendocrine (NET)/carcinoid tumors are less commonly studied and have only been described in small series. The purpose of this study was to describe the demographics and natural history of esophageal NETs, as well as optimal treatments. We hypothesized that surgical resection would be the best treatment of esophageal NETs. The National Cancer Database was used to identify adult patients with esophageal or gastroesophageal junction (GEJ) cancer from 2004 to 2018. Patients were characterized as carcinoid/NET, adenocarcinoma, or squamous cell cancer. Clinical and demographic characteristics were compared between the histology groups. The primary outcome was overall survival, which was assessed by multivariable Cox analysis. Multivariable Cox analysis was also used to analyze factors associated with survival among NET patients who underwent surgery. Among 206,321 patients with esophageal cancer, 1,563 were NETs (<0.01%). Relative to the other two histologies, NETs were associated with younger age, female sex, and advanced clinical stage at diagnosis. Multivariate analysis suggested that NETs were less likely to be treated with surgical resection (OR 0.51, P<0.001). Nonetheless, surgical resection was associated with improved survival (HR 0.64, P=0.003). Among patients with NETs who received surgery, neoadjuvant therapy was associated with improved overall survival (HR 0.38, P=0.013). NET of the esophagus presents with more advanced disease than other common histologies. Among patients with nonmetastatic cancer, surgical resection appears to be the best treatment. Neoadjuvant systemic therapy may offer survival benefit, but future studies are necessary.